Lysosomal enzyme

A kind of biological enzyme

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lysosome Enzymes are Biological enzyme One of. Lysosome is a series of lysosomes in the cytoplasm surrounded by a single layer of lipoprotein membrane acid hydrolase Corpuscle of. Lysosomes are cells with a single-layer membrane like structure Organelle , lysosomes contain many kinds of Hydrolases It can decompose many substances, and lysosomes are compared to "enzyme warehouses" in cells“ digestive system ”。

Chinese name: Lysosomal enzyme
Number: About 60 kinds

Properties: Biological enzyme
Category: Hydrolases

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Lysosomal enzyme

Enzymes in lysozyme are hydrolase , and general Optimum pH It is 5, so they are all acid hydrolases. If the enzyme in lysozyme is released, the whole cell will be digested. Generally not released to internal environment , mainly Intracellular digestion 。

Lysosomal enzyme

Types of lysosomal enzymes

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It is known that lysosomes of various cells contain about 60 enzymes, including protein , sugar lipid Isocratic Hydrolases , such as acid Phospholipase 、 cathepsin 、 Ribonuclease And aromatic sulfatases A and B. The hydrolases in various lysosomes are also different. The enzymes in most lysosomes are glycoprotein , but there are exceptions, such as Rat liver Cells and Renal cell Most of the enzymes in lysosomes are Lipoprotein 。 Common lysosomal enzymes include: Aryl sulfatase A(ASA)、 Galactose Cerebrosidase β - galactosidase 、β- amino Already Glycoside Enzyme A, total Hexosaminidase 、 β - glucosidase , sphingomyelinase, α- Galactosidase 、α- Glucosidase 、α- Fucosidase 、α- Mannosidase , acidic β - mannosidase, asparagus Aminoacyl amino Glucose Glycosidase, α - L-Edu glycosidase Eduronic acid Sulfatase 、 Acetyl heparin - N - sulfuric acid esterase 、α-N- Acetylamino Glucosidase, acetyl CoA-glucosamine - N- Acetyltransferase , N-acetylglucosamine 6-sulfatase, galactose-6-sulfatase, β - galactosidase, aryl sulfatase, β -- Glucuronic acid Glycosidase Hyaluronidase 、 Phosphotransferase , palmitoyl protein thiolipase Carboxypeptidase (TPP1 lysosome Peptidase )、 Ceramidase 、 Sialidase , acid esterase, etc.

Lysosomal enzymes and diseases

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Lysosomal enzymatic activity Insufficient (mainly acid hydrolase) Activator protein 、 Transporter Or lysosomal protein processing corrects the lack of enzyme, which causes lysosome Functional defect , causing Secondary lysosome The corresponding substrate cannot be digested, and the substrate is accumulated, Metabolic disorder , forming a storage disease, called Lysosomal storage disease Lysosomal storage disease not only affects the normal function of one organ of the body, but also often affects the metabolic activity of the whole body Coordination , causing a variety of diseases. At present, there are more than 40 kinds of such diseases, which can be roughly divided into Glycogen accumulation disease 、 Cerebrosidosis , Tai Sa syndrome, and Mucopolysaccharide deposition disease And so on^[1] 。

Glycogen accumulation disease

Glycogen accumulation disease (glycogen storage disease, GSD) muscle cells Lacking an acid Ⅱ - Glucosidase 。 This enzyme decomposes normally Glycogen , and this Enzyme deficiency The excess glycogen swallowed by lysosome cannot be degraded, and a large amount of glycogen is piled up in the secondary lysosome to make it swell, eventually leading to lysosome rupture and other enzymes leakage, Severe damage Histiocyte 。 This disease belongs to Autosome Defect Hereditary disease , most of the patients are children, who often die before the age of two^[1] 。

Cerebrosidosis

Cerebrosidosis (cerebrosidosis), also known as Gaucher disease（ Gaucher disease ）, Yes macrophage And brain nerve cell Lysosome deficiency β- glucose Glycosidase. Large amount of glucose Cerebroside Deposited in these lysosomes, macrophages become Gaucher cells, and patients develop liver Splenomegaly ， Thrombocytopenia ， anemia , bone pain and other symptoms, serious occurrence Ocular Motility Disorders ， Ataxia etc. Central nervous system symptoms 。 This disease occurs at all ages, ranging from infants to adults. The severity of symptoms varies greatly. The earlier the disease occurs, the more severe the symptoms are prognosis The worse.^[1] 。

Tay-Sachs diesease

Tay Sachs syndrome: lysosome lacks β - N-hexosaminidase, resulting in GM2 Ganglioside It cannot be hydrolyzed and stored in lysosomes, thus causing functional damage to cells. The incidence of the disease is the highest among German Jews, which is very rare in China. It shows familial dementia, brain macular degeneration 。 The damage of nerve cells is more obvious in this disease. Therefore, Nervous tissue dysfunction It is outstanding, as shown in Gradualness Blindness, dementia and paralysis. Only two cases have been reported in China so far^[1] 。

Mucopolysaccharide deposition disease

Mucopolysaccharide deposition disease (mucopolysaccharidosis) is a group of Mucopolysaccharide Genetic disease of progressive metabolic disorder. The pathology is that there is no mucopolysaccharide degrading enzyme in the lysozyme body, so it cannot decompose mucin Polysaccharides And make these substances accumulate in the secondary lysosome. The patient has rough face, abnormal skeleton and intelligence Hypoplasia , visceral function is generally damaged, Corneal opacification 。 Glycosaminoglycans were deposited in the body of the child, especially in the brain, heart, liver and spleen. Cornea, bone tendon Secondly, the heart is mainly affected by valves and myocardium. clinical manifestation There are also differences. At present, there are 7 types^[1] 。

dissolve Enzyme body The method of lysosomal enzyme activity detection can be used to measure the enzyme loss, insufficiency or poor activity. The lysosomal enzyme activity detection is the confirmation of the enzyme body Mannosidosis Of gold standard 。 Domestic Peking Union Medical College Hospital 、 Shanghai Xinhua Hospital Beijing Zhongke medical laboratory The lysosomal enzyme activity can be detected by other units.

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